What is Batten disease?
What is Batten disease?
Batten disease is a group of fatal genetic disorders. There are 13 types. The disorder affects the body’s ability to get rid of cellular waste (lipids and proteins), so they build up in cells all over the body. The buildup causes seizures, vision loss, problems with thinking and movement, and eventually, death.
Is Battens disease a metabolic disease?
Inherited metabolic disorder Batten disease is an inherited disorder in which both parents of a patient are carriers of the disease but are not themselves ill. Both parents pass on one chromosome with the disease. Children of these parents have a 25% chance to get the disease.
What is Juvenile Batten Disease?
CLN3, often called juvenile Batten disease, is an ultra-rare, fatal, inherited disorder that primarily affects the nervous system and left untreated, is fatal. Children with CLN3 disease develop normally, even excelling in school until ages 5–6 years, when progressive vision loss becomes noticeable.
Why does Batten disease happen?
Batten disease is caused when both copies (one from each parent) of the specific gene causing the disease are defective. This is known as autosomal recessive disease. People who only have one defective copy (carriers) will not develop symptoms and are usually unaware of their carrier condition.
How does Batten disease happen?
Batten disease is a rare group of nervous system disorders called neuronal ceroid lipofuscinosis (NCLs) that get worse over time. It usually starts in childhood, between the ages of 5 and 10. There are different forms of the disease but all are fatal, usually by the late teens or twenties.
What is infantile Batten disease?
Batten disease is a fatal, inherited disorder of the nervous system that begins in childhood.
What is diagnosis code 10?
ICD-10-CM stands for International Classification of Diseases , 10th Edition, Clinical Modification, and is used for diagnosis coding. The format of ICD-10-CM codes is very similar to ICD-9-CM codes, but the new system uses 3-7 alpha and numeric characters and full code titles for each code.
